Authors: Kwiatkowski, Janet L.¹; Granger, Suzanne²; Brambilla, Donald J.²; Brown, R. Clark³; Miller, Scott T.⁴; Adams, Robert J.⁵

Source: British Journal of Haematology, Volume 134, Number 3, August 2006 , pp. 333-339(7)

Publisher: Wiley-Blackwell

Abstract:

Summary

Elevated velocity in the internal carotid artery (ICA) or middle cerebral artery (MCA), detected by transcranial Doppler (TCD) ultrasonography, predicts an increased risk of stroke in children with sickle cell disease (SCD). Although strokes also occur in an anterior cerebral artery (ACA) distribution, the significance of elevated velocity in this vessel has not been determined previously. We assessed the effect of elevated ACA velocity on stroke risk using the results of the first adequate TCD study performed on 1975 children as part of The Stroke Prevention Trial in Sickle Cell Anemia (STOP). Elevated ACA velocity (≥170 cm/s) was associated with an increased risk of stroke (P = 0·0013) after adjusting for the ICA/MCA classification. Among subjects with normal ICA/MCA velocity, the risk of stroke was more than 10-fold greater in those with elevated compared with normal ACA velocity (2·13 and 0·20 per 100 patient-years, respectively, P < 0·001); risk more than doubled with elevated compared with normal ACA velocity in those already at high risk due to abnormal ICA/MCA findings (7·56 vs. 3·22 per 100 patient-years, P = 0·042). Few of the strokes in those with elevated ACA velocity occurred in an ACA distribution, suggesting changes in blood flow velocity in anterior vessels may be associated with diffuse arterial disease or, alternatively, manifest collateral flow from compromised middle cerebral vessels.

Keywords: sickle cell disease; stroke; child; sickle cell radiology; vascular disease

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1365-2141.2006.06193.x

Affiliations: 1: Division of Hematology, The Children's Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 2: New England Research Institutes, Watertown, MA 3: Children's Healthcare of Atlanta/Emory University, Atlanta, GA 4: SUNY-Downstate Medical Center/Kings County Hospital, Brooklyn, NY 5: Medical College of Georgia, Augusta, GA, USA

Publication date: 2006-08-01

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